PDF(Pubmed)
Abstract：
Dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel by genetic mutations causes the inherited disease cystic fibrosis (CF). CF lung disease that involves multiple disorders of epithelial function likely results from loss of CFTR function as an anion channel conducting chloride and bicarbonate ions and its function as a cellular regulator modulating the activity of membrane and cytosol proteins. In the absence of CFTR activity, abundant mucus accumulation, bacterial infection and inflammation characterize CF airways, in which inflammation-associated tissue remodeling and damage gradually destroys the lung. Deciphering the link between CFTR dysfunction and bacterial infection in CF airways may reveal the pathogenesis of CF lung disease and guide the development of new treatments. Research efforts towards this goal, including high salt, low volume, airway surface liquid acidosis and abnormal mucus hypotheses are critically reviewed.
摘要：
遗传突变引起的囊性纤维化跨膜传导调节因子（CFTR）阴离子通道的功能障碍导致遗传性疾病囊性纤维化（CF）。涉及多种上皮功能紊乱的CF肺病可能是由于CFTR作为传导氯离子和碳酸氢根离子的阴离子通道的功能丧失及其作为调节膜和细胞溶胶蛋白活性的细胞调节剂的功能丧失所致。在没有CFTR活性的情况下，丰富的粘液积累，细菌感染和炎症表征CF气道，其中炎症相关的组织重塑和损伤逐渐破坏肺。破译CFTR功能障碍与CF气道细菌感染之间的联系可能揭示CF肺病的发病机制并指导新的治疗方法的发展。为实现这一目标而努力的研究，包括高盐，低音量,严格审查了气道表面液体酸中毒和异常粘液假设。
